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CERTAINTY OF GOALS: WHEN
THE OUTCOME IS UNCERTAIN
FOR CHILDREN WITH SEVERE
NEUROLOGICAL IMPAIRMENTJulie Hauer, MD
Ireland’s 4th International
Children’s Palliative Care
Conference, 22 Nov, 2018
Objectives
Discuss problems due to severe impairment of the central nervous system that can remain intractable to treatment options
Define tipping points that can prompt anticipatory discussions, including suggested recommendations that best meet goals
Utilize language strategies that assist families when prognosis is not possible
Courageous
Parents
Network:
videos with
his mother
o Prognosis: prediction of probable course
o Wide range of outcomes
o 2 survival phases at birth or injury
o No biomarkers
o Goal: lessen decisional regret
Challenges with Decision Making
Leong K 2013, Merker B 2008, Whiteford 1996
Outcome based (death, development) and Decision
based (surgery, DNI/DNR)
Process based (define goals,
review options, attend to
emotions, allow hope)
Prognosis Palliative Care
Uncertainty to Certainty
Uncertainty of Outcome: life span, recovery,
benefit from intervention
Certainty of Decision:
purpose, intent, and goal of a
decision
Bogetz, JF, Hauer J. 2018.
o Adaptive: to cope with
adversity and loss
o Affirmative: to convey
commitment and love
Feudtner 2009, Feudtner et al 2010
Hope
I’ve learned from
my son that
everything is
possible, it doesn’t
mean everything
will happen.
Quality of Life (QOL) Considerations
QOL as indicated by parents of 14 children with profound intellectual and motor disability (PIMD)
Ability to enjoy
Comfort
Awareness
Multiple health problems
Falling below the lower threshold of QOL
Zaal-Schuller IH, et al. Considering quality of life in end-of-life decisions for severely
disabled children. Res Dev Disabil. 2018;73:67-75.
End of Life Decision Making (EOLD)
Parents: half indicated missed opportunity by
physicians to discuss EOLD at time of stability
Physicians: found it difficult to identify when a
parent was “ready” for discussion
Physician discussion: typically initiated at
time of acute deterioration
Zaal-Schuller IH, et al. How parents and physicians experience end-of-life decision-making for
children with profound intellectual and multiple disabilities. Res Dev Disabil. 2016;59:283-293.
Patterns of End of Life Care
Progressive disorders of the CNS versus Static encephalopathy, congenital, genetic
oStatic group less likely to have palliative care consult, die at home, plan location of death, or have DNR orders in place
oStatic more likely to receive intensive life sustaining therapies in last 2 days of life
DeCourcey DD, et al. 2018.
34 deaths: general characteristics
Average age: 22 years
Age range: 11 months to 50 years
10 (29%) older than 30 years
Conditions: anoxic and traumatic brain injury, neuro-structural malformations, in-utero infection, genetic, metabolic
Hauer J, et al. Anticipating Death in Children and Adults with Childhood Onset Severe Central Nervous
System Impairment: A Case Series Review (FR480). J Pain Symptom Manage. 2018;55(2):631.
General Characteristics
Feature / Problem N=34
Feeding tubes N=34 (100%)
Anti-seizure drugs N=33 (97%)
Complex symptom management N=23 (68%)
Respiratory: chronic, recurrent N=15 (44%)
Gastrointestinal: acute recurrent N=10 (29%)
Ventilation: invasive, noninvasive N=7 (21%)
Goals Discussion N=27 (79%)
Triggers: symptoms, decreased engagement
in activities, health burden, EOL features
Triggers added: functional ileus
Common goals:To be comfortable, To not sufferTo get better, To live as long as possibleTo be with family and loved ones
Redirection of Care in 23 of the 27
Reasons for Redirection N= 23 of 34
General decline N=19 (55%)
Global symptom burden N=16 (47%)
End of life (EOL) features N=16 (47%)
Respiratory: chronic, recurrent N=15 (44%)
Process: days, months, years
Time until death: days to 5 years
Quality of Life and Goals of Care
Natural tension between problems without cure
that we intend to improve though may not
have the hoped for outcome
Thriving Declining EOLDesired heath Quality of Life
and function Goals of CareModifiable?
Iterative Discussion Loop
Trigger
Goals
Options
Reflect Revisit
Triggers for meeting
Significant changes
Symptoms
Quality of life
Health
End-of-life features
Edema
Functional ileus
Ileus or Edema at End of Life
Ileus and/or edema at End-of-Life: 13 (38%)o Acute or recurrent ileus: N=10 (29%)
o Edema: N=6 (18%)
Assessment: metabolic panel, urine analysis and culture
o 11: pre-existing decline in health, function, QOL
o 2 with severe anoxic brain injury
Ileus with recovery: stable QOL and health
Siden H, et al. Pediatric enteral feeding intolerance: a new prognosticator for children with life-limiting illness?
Hauer J. Feeding Intolerance in Children with Severe Impairment of the Central Nervous System
Language and Pauses to Listen
“These features worry me…”
“The tests were normal. It is unlikely that there is
a fixable reason for his intestines shutting down.
This can be due to the body shutting down or
changes in the area of the brain that regulates
the gut. In some, gut function won’t return.”
Hauer J. Feeding Intolerance in Children with Severe Impairment of the Central Nervous
System: Treatment and Prevention. Children (Basel). 2017; 5(1). pii: E1.
Language and Pauses to Listen
“We will give an amount of fluid that his body is
more likely to tolerate. We will discuss what that
means if he doesn’t…”
“I’m glad we are protecting him from
feeds/fluids that his body can’t process at this
time”
Decision to hold feeds/fluids: no regret, “lesser
of 2 evils”, “the only thing that made any sense”Rapoport A, et al. Pediatrics. 2013;131(5):861-9.
Neurophysiology is Complex
Hypothalamus, Thalamus, Midbrain,
Pons, Medulla regulation✓Heart rate, blood pressure, temperature
✓Arousal
✓Vasomotor (hypothalamus, medulla)
✓ Intestinal motility, vomiting
✓ Respiratory center
End-of-Life (EOL) Decision Making
“I don’t believe in mights.” Does not help to
consider “what might happen”
Mom’s coping: focus on how well he will do
Revisit: if goal of comfort cannot be met or
intervention will not meet physiological goal
An Official ATS/AACN/ACCP/ESICM/SCCM Policy Statement: Responding to Requests for
Potentially Inappropriate Treatments in Intensive Care Units
Goal Concordant EOL Care
Need for around the clock nursing
Requires experts in EOL care
Active adjustments in care plan
Symptom management
Adjust feeds / fluids at end of life
Stop nonessential interventions
A Point in Time
TIME = days, weeks, months, years
HEA
LTH
/ Q
ualit
y o
f Li
fe
HOPE2 trajectories at the beginning
o Hours to weeks, occasionally months
o Those who live months often live years
Time to “relearn” their child
Create an explicit process for triggers
What goals are intended by the intervention
Life extending and…
See what outcome is possible, attempt to modify
health and/or quality of life
Celebrate success!
Patience, reflect and revisit goals as needed
What can we “measure”
Goals of Care – the Horizon
When did we go from assisting with his
breathing to insisting that he breathe
The line that divides the visible
from the invisible
Conclusions and considerations
Define triggers: monitor for changes in
quality of life and burden of treatment
Determine Goals of Care, revisit as needed
Offer goal concordant treatment plans
End-of-life care requires expertise and
intermittent around the clock nursing
References and Further Reading
Allen KA. Parental decision-making for medically complex infants and children: an integrated literature review. Int J Nurs Stud. 2014;51(9):1289-304.
Bogetz, JF, Hauer J. Certainty of Decisions: A Process-Based Model for Decision Making for Children with Severe Neurological Impairment. ClinPediatr (Phila). 2018;57(10):1227-1231
DeCourcey DD, et al. Patterns of Care at the End of Life for Children and Young Adults with Life-Threatening Complex Chronic Conditions. J Pediatr. 2018;193:196-203.e2. doi: 10.1016/j.jpeds.2017.09.078.
Feudtner C, et al. Good-parent beliefs of parents of seriously ill children. JAMA Pediatr. 2015;169(1):39-47.
Feudtner C. The breadth of hopes. N Engl J Med. 2009;361(24):2306-7.
Feudtner C, et al. Parental hopeful patterns of thinking, emotions, and pediatric palliative care decision making: a prospective cohort study. Arch Pediatr Adolesc Med. 2010;164(9):831-9.
References and Further Reading
Feudtner C. Collaborative communication in pediatric palliative care: a foundation for problem-solving and decision-making. Pediatr Clin North Am. 2007;54(5):583-607, ix.
Hauer J, Houtrow A. Pain Assessment and Treatment in Children With Significant Impairment of the Central Nervous System. Pediatrics. 2017;139(6):e20171002
Hauer J, Clark C, Jarek H. Anticipating Death in Children and Adults with Childhood Onset Severe Central Nervous System Impairment: A Case Series Review (FR480). J Pain Symptom Manage. 2018;55(2):631.
Hauer J. Feeding Intolerance in Children with Severe Impairment of the Central Nervous System: Treatment and Prevention. Children (Basel). 2017; 5(1). pii: E1. doi: 10.3390/children5010001.
Hauer J. Caring for Children who have Severe Neurological Impairment: A Life with Grace. Baltimore, Maryland: Johns Hopkins University Press, 2013, pp 164-177, 328-338.
References and Further Reading
Hill DL, et al. Regoaling: a conceptual model of how parents of children with serious illness change medical care goals. BMC Palliat Care. 2014;13(1):9.
Siden H, et al. Pediatric enteral feeding intolerance: a new prognosticator for children with life-limiting illness? J Palliat Care. 2009;25(3):213-7.
Tamburro RF, et al. Care goals and decisions for children referred to a pediatric palliative care program. J Palliat Med. 2011;14:607-613.
Zaal-Schuller IH, et al. How parents and physicians experience end-of-life decision-making for children with profound intellectual and multiple disabilities. Res Dev Disabil. 2016;59:283-293.
Zaal-Schuller IH, et al. Considering quality of life in end-of-life decisions for severely disabled children. Res Dev Disabil. 2018;73:67-75.
An Official ATS/AACN/ACCP/ESICM/SCCM Policy Statement: Responding to Requests for Potentially Inappropriate Treatments in Intensive Care Units: https://www.thoracic.org/statements/resources/cc/inappropr-ther-st.pdf
Quality of Life (QOL) Considerations
Feudtner C. Collaborative communication in pediatric palliative care: a foundation for
problem-solving and decision-making. Pediatr Clin North Am. 2007;54(5):583-607, ix.
Respiratory Features
Respiratory distress: active respiratory effort and unmet symptom burden, uncommon at EOL
Opioid use in 15: 13 (86%) on scheduled morphine: months to years
“I think he lived longer because of this”
Acute and chronic treatment
Change in respiratory rate and rhythm at EOL: apneic pauses, Cheyne-Stokes, agonal
Goals of Care and Decision Making
Altered QOL (alertness, comfort)
Hopes/Goals: improved health,
alertness, and comfort
“I’ve heard you mention several hopes,
let’s review what might be possible”
• Follow-up: “I wish…” “I wonder…”
• Discussion when technology not
offering the benefit it once did
• “I’m glad for all the years of benefit…”
• Introduced with no need for decision
Goals of Care and Decision Making
Language Suggestions
Acknowledge the emotion: I see your distress, I
am so sorry for how hard this is for you.
Attend to the concern: I’m glad we are
protecting him from the cause of his discomfort.
Redirect to an achievable goal: His face looks
so relaxed, do you have the same observation?
